Pmp22+/- mice were backcrossed with
C57Bl6J mice (Jackson Lab) for more than 10 generations to reach congenic. Mice were genotyped as described [9 (
link)]. The primers for genotyping are listed in
S3 Table. The congenic
Pmp22+/- mice have been extensively characterized [5 (
link)]. They showed pathology and other features similar to those in
Pmp22+/- mice with mixed background (C57Bl6J/129) [10 (
link)].
Pak1-/- mice were from Dr. Jonathan Chernoff’s lab, Fox Chase Cancer Center, USA. The
Pak1-/- mice were produced in C57Bl6J background and have been described with negligible phenotype [19 (
link)].
For PAK1 inhibitor (PF3758309) injections,
Pmp22+/- mice were randomized into vehicle and treated groups. Based on our power calculation of variations derived from mouse compound nerve action potentials, 7 mice for each group would have a 92% chance to detect a significant difference.
PF-3758309 (Cat# CT-PF0375, ChemieTek) was dissolved in normal saline for intraperitoneal injection (i.p.) daily.
Hu B., Arpag S., Zhang X., Möbius W., Werner H., Sosinsky G., Ellisman M., Zhang Y., Hamilton A., Chernoff J, & Li J. (2016). Tuning PAK Activity to Rescue Abnormal Myelin Permeability in HNPP. PLoS Genetics, 12(9), e1006290.